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Childhood hearing loss

Ryan H Belcher, David W Molter, Steven L Goudy
Despite humanitarian otolaryngology groups traveling in record numbers to resource-limited areas treating pediatric otolaryngology disease processes and training local providers, there remains a large burden of unmet needs. There is a meager amount of published information that comes from the developing world from an otolaryngology standpoint. As would be expected, the little information that does comes involves some of the most common pediatric otolaryngology diseases and surgical burdens including childhood hearing loss, otitis media, adenotonsillectomies, airway obstructions requiring tracheostomies, foreign body aspirations, and craniomaxillofacial surgeries, including cleft lip and palate...
March 7, 2018: Otolaryngologic Clinics of North America
Caroline Jones, Mridula Sharma, Samantha Harkus, Catherine McMahon, Mele Taumoepeau, Katherine Demuth, Karen Mattock, Lee Rosas, Raelene Wing, Sulabha Pawar, Anne Hampshire
BACKGROUND: Indigenous infants and children in Australia, especially in remote communities, experience early and chronic otitis media (OM) which is difficult to treat and has lifelong impacts in health and education. The LiTTLe Program (Learning to Talk, Talking to Learn) aimed to increase infants' access to spoken language input, teach parents to manage health and hearing problems, and support children's school readiness. This paper aimed to explore caregivers' views about this inclusive, parent-implemented early childhood program for 0-3 years in an Aboriginal community health context...
March 6, 2018: BMC Pediatrics
M K Pein, T Rahne, A Noll, S K Plontke
No abstract text is available yet for this article.
March 2, 2018: HNO
Marc A Brennan, Ryan W McCreery, Emily Buss, Walt Jesteadt
OBJECTIVES: The objective of this experiment was to examine the contributions of audibility to the ability to perceive a gap in noise for children and adults. Sensorineural hearing loss (SNHL) in adulthood is associated with a deficit in gap detection. It is well known that reduced audibility in adult listeners with SNHL contributes to this deficit; however, it is unclear the extent to which hearing aid amplification can restore gap-detection thresholds, and the effect of childhood SNHL on gap-detection thresholds have not been described...
February 27, 2018: Ear and Hearing
Mun Young Chang, Chung Lee, Jin Hee Han, Min Young Kim, Hye-Rim Park, Nayoung Kim, Woong-Yang Park, Doo Yi Oh, Byung Yoon Choi
BACKGROUND: MYO15A variants, except those in the N-terminal domain, have been shown to be associated with congenital or pre-lingual severe-to-profound hearing loss (DFNB3), which ultimately requires cochlear implantation in early childhood. Recently, such variants have also been shown to possibly cause moderate-to-severe hearing loss. Herein, we also demonstrate that some MYO15A mutant alleles can cause postlingual onset of progressive partial deafness. METHODS: Two multiplex Korean families (SB246 and SB224), manifesting postlingual, progressive, partial deafness in an autosomal recessive fashion, were recruited...
February 27, 2018: BMC Medical Genetics
A Smith, A O'Connor, S Hennessy, P G O'Sullivan, L Gibson
The Newborn Hearing Screening Programme (NHSP) was established in Cork University Maternity Hospital (CUMH) in April 2011. Between April 2011 and July 2014, 42 infants were identified with a Permanent Childhood Hearing Impairment (PCHI). Following this diagnosis, infants underwent a paediatric assessment according to recognised guidelines with the intention of identifying the underlying aetiology of the PCHI. The aim of this study was to assess the findings of this aetiological workup via retrospective chart review...
December 18, 2017: Irish Medical Journal
Chae-Hyun Lim, Ji Hyung Lim, Doyoun Kim, Ho Sung Choi, Dong-Hee Lee, Dong-Kee Kim
OBJECTIVES: This study was performed to evaluate the frequency of bony cochlear nerve canal (BCNC) stenosis and its clinical significance in pediatric patients with unilateral sensorineural hearing loss (SNHL) of unknown etiology. MATERIALS AND METHODS: We analyzed the medical records and temporal bone computed tomography (CT) results of patients less than 13 years of age with a diagnosis of unilateral SNHL of unknown etiology between July 2007 and July 2017. We compared the BCNC diameter between both sides and analyzed the age at diagnosis, degree of hearing loss, and accompanying inner ear anomalies...
March 2018: International Journal of Pediatric Otorhinolaryngology
Shouneez Yousuf Hussein, De Wet Swanepoel, Leigh Biagio de Jager, Faheema Mahomed-Asmail
OBJECTIVE: Within the educational sector of low-and middle-income countries (LMICs), formal and informal early childhood development (ECD) centers are often the first point of contact for majority of children. Since early hearing detection services are mostly absent in LMICs, these ECD centers may serve as the first point of access to screenings for these children. ECD practitioner awareness regarding hearing and hearing loss is essential for the successful implementation of hearing screening programs...
March 2018: International Journal of Pediatric Otorhinolaryngology
Maggie Yee Yan Lam, Eddie Chi Ming Wong, Chi Wai Law, Helena Hui Ling Lee, Bradley McPherson
OBJECTIVES: To facilitate early diagnosis of infants with hearing loss, a universal newborn hearing screening program (UNHS) has been implemented in Hong Kong's public hospitals for over a decade. However, there have been no known studies investigating parent attitudes to, and satisfaction with, UNHS since its launch in Hong Kong. The present study aimed to investigate knowledge of UNHS as well as infant hearing development, and attitudes and satisfaction with UNHS, in Hong Kong mothers with newborns...
February 2018: International Journal of Pediatric Otorhinolaryngology
Susan D Emmett, Jane Schmitz, Sureswor L Karna, Subarna K Khatry, Lee Wu, Steven C LeClerq, Joseph Pillion, Keith P West
Background: Prevalence of young adult hearing loss is high in low-resource societies; the reasons for this are likely complex but could involve early childhood undernutrition. Objective: We evaluated preschool childhood stunting, wasting, and underweight as risk factors for hearing loss in young adulthood in Sarlahi District, southern Nepal. Design: Ear health was assessed in 2006-2008 in a cohort of 2193 subjects aged 16-23 y, who as children <60 mo of age participated in a 16-mo placebo-controlled, randomized vitamin A supplementation trial from 1989 to 1991...
February 7, 2018: American Journal of Clinical Nutrition
Tommaso Schirinzi, Federica Graziola, Francesco Nicita, Lorena Travaglini, Fabrizia Stregapede, Massimiliano Valeriani, Paolo Curatolo, Enrico Bertini, Federico Vigevano, Alessandro Capuano
ATP1A3 mutations are related to a wide spectrum of clinical conditions, including several defined syndromes as rapid-onset dystonia-parkinsonism (RDP), alternating hemiplegia of childhood (AHC), and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS), together with many other intermediate phenotypes. Ataxia is always more increasingly reported, either as accessory or prominent sign, in ATP1A3-related conditions, being thus considered as a peculiar feature of this spectrum...
February 3, 2018: Cerebellum
Michela Stagnaro, Livia Pisciotta, Marcella Gherzi, Maja Di Rocco, Fiorella Gurrieri, Elena Parrini, Giulia Prato, Edvige Veneselli, Elisa De Grandis
Mutations in the ATP1A3 gene, which encodes the alpha3-subunit of sodium-potassium ATPase, are related to a spectrum of neurological diseases including Rapid onset Dystonia-Parkinsonism (RDP), Alternating Hemiplegia of Childhood (AHC) and Cerebellar ataxia, Areflexia, Pes cavus, Optic atrophy and Sensorineural hearing loss (CAPOS) syndrome. Moreover, an increasing number of patients with intermediate and non classical phenotypes have been reported. Herein we describe 7 patients with 6 different de novo ATP1A3 mutations, and we focus on paroxysmal and chronic movement disorders with the help of video documentation...
January 29, 2018: European Journal of Paediatric Neurology: EJPN
Jing Wang, Carlijn M P le Clercq, Valerie Sung, Peter Carew, Richard S Liu, Fiona K Mensah, Rachel A Burt, Lisa Gold, Melissa Wake
OBJECTIVE: In a national study of Australian children aged 11-12 years old, we examined the (1) prevalence and characteristics of hearing loss, (2) its demographic risk factors and (3) evidence for secular increases since 1990. METHODS: This is a cross-sectional CheckPoint wave within the Longitudinal Study of Australian Children. 1485 children (49.8% retention; 49.7% boys) underwent air-conduction audiometry. Aim 1: hearing loss (≥16 decibels hearing level (dB HL)) was defined in four ways to enable prior/future comparisons: high Fletcher Index (mean of 1, 2 and 4 kHz; primary outcome relevant to speech perception), four-frequency (1, 2, 4 and 8 kHz), lower frequency (1 and 2 kHz) and higher frequency (4 and 8 kHz); aim 2: logistic regression of hearing loss by age, gender and disadvantage index; and aim 3: P for trend examining CheckPoint and reported prevalence in studies arranged by date since 1990...
January 31, 2018: Archives of Disease in Childhood
Miranda L Camet, Susan S Hayashi, Belinda C Sinks, Jennifer Henry, Katie Gettinger, Ashley Hite, Juliann Kiefer, Caroline Mohrmann, Taryn Sandheinrich, Feng Gao, Robert J Hayashi
BACKGROUND: Sensorineural hearing loss due to ototoxic cancer therapy is well established; effects on the vestibular system are unknown. We examined the feasibility of implementing vestibular screens for pediatric cancer survivors exposed to ototoxic agents. The prevalence of screening failures is reported. METHODS: Cancer survivors who were 6-17 years, at least 1-month posttreatment, and received ototoxic therapy (radiation to the head/neck, cisplatin, carboplatin) were eligible...
January 30, 2018: Pediatric Blood & Cancer
Michael Hoffman, Elena Tiddens, Alexandra L Quittner
OBJECTIVE: Previous research has found that preschoolers with hearing loss have worse visual attention and elevated rates of behavior problems when compared to typically hearing peers (Barker et al., 2009). However, little is known about these deficits in school-age children with cochlear implants (CIs). We evaluated visual selective attention in school-age children with CIs and hearing peers and examined the link between visual attention and behavior problems. METHOD: Data were drawn from the Childhood Development after Cochlear Implantation (CDaCI) study, the largest longitudinal, multi-site study of children with CIs...
January 9, 2018: Hearing Research
Hazel I Blythe, Jonathan H Dickins, Colin R Kennedy, Simon P Liversedge
There has been considerable variability within the literature concerning the extent to which deaf/hard of hearing individuals are able to process phonological codes during reading. Two experiments are reported in which participants' eye movements were recorded as they read sentences containing correctly spelled words (e.g., church), pseudohomophones (e.g., cherch), and spelling controls (e.g., charch). We examined both foveal processing and parafoveal pre-processing of phonology for three participant groups-teenagers with permanent childhood hearing loss (PCHL), chronological age-matched controls, and reading age-matched controls...
January 22, 2018: Developmental Science
Julia Gunkel, Linda S de Vries, Marian Jongmans, Corine Koopman-Esseboom, Ingrid C van Haastert, Maria C J Eijsermans, Carolien van Stam, Bert G A van Zanten, Tom F W Wolfs, Joppe Nijman
OBJECTIVES: To assess whether preterm infants with postnatal cytomegalovirus infection develop neurologic sequelae in early childhood. METHODS: Infants <32 weeks' gestation were prospectively screened for cytomegalovirus (CMV) at term-equivalent age. Neurodevelopment was compared between CMV-positive and CMV-negative infants by using the Griffiths Mental Development Scales (GMDS) at 16 months' corrected age (CA); the Bayley Scales of Infant and Toddler Development, Third Edition or the GMDS at 24 to 30 months' CA; and the Wechsler Preschool and Primary Scale of Intelligence, Third Edition and Movement Assessment Battery for Children, Second Edition at 6 years of age...
February 2018: Pediatrics
Jun Okada, Mohammad Arif Hossain, Chen Wu, Takashi Miyajima, Hiroko Yanagisawa, Keiko Akiyama, Yoshikatsu Eto
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of α-galactosidase A (α-gal A), leading to the progressive accumulation of glycosphingolipids. Classical hemizygous males usually present symptoms, including pain and paresthesia in the extremities, angiokeratoma, hypo- or anhidrosis, abdominal pain, cornea verticillata, early stroke, tinnitus, and/or hearing loss, during early childhood or adolescence. Moreover, proteinuria, renal impairment, and cardiac hypertrophy can appear with age...
March 2018: Molecular Genetics and Metabolism Reports
Alina Henn, Harald Weng, Simon Novak, Günther Rettenberger, Andreas Gerhardinger, Eva Rossier, Birgit Zirn
Heterozygous microdeletions of chromosome 2p21 encompassing only the SIX2 gene have been described in two families to date. The clinical phenotype comprised autosomal-dominant inherited frontonasal dysplasia with ptosis in one family. In the second family, conductive hearing loss was the major clinical feature described; however, the affected persons also had ptosis. Here, we present a large family combining all three predescribed features of SIX2 gene deletion. The phenotype in four affected family members in three generations consisted of bilateral congenital ptosis, epicanthus inversus, frontonasal dysplasia with broad nasal bridge and hypertelorism, frontal bossing and large anterior fontanel in childhood, narrow ear canals, and mild conductive hearing loss with onset in childhood...
January 8, 2018: Clinical Dysmorphology
Stéphanie Paquay, Elsa Wiame, Naima Deggouj, Antonella Boschi, Romolo Daniele De Siati, Yves Sznajer, Marie-Cécile Nassogne
CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is a rare neurological disorder, recently associated with the c.2452G > A hotspot mutation in the ATP1A3 gene, with sensorineural hearing loss as a prominent feature. We herein report on a girl who has experienced hearing loss for three years following an initial encephalitic episode when aged 15 months old. CAPOS was diagnosed only when she was six years old by targeted testing whilst she displayed optic atrophy, cerebellar signs and areflexia...
January 2018: International Journal of Pediatric Otorhinolaryngology
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